Pancreatic Solid Pseudopapillary Neoplasm Research Articles

Surgical Outcomes of Pancreatic Solid Pseudopapillary Neoplasm: Experiences of 24 Patients in a Single Institute

Background and Objectives: The pancreatic solid pseudopapillary neoplasm (SPN), a rare tumor predominantly affecting young women, has seen an increased incidence due to improved imaging and epidemiological knowledge. This study aimed to understand the outcomes of different interventions, possible complications, and associated risk factors. Materials and Methods: This study retrospectively analyzed 24 patients who underwent pancreatic surgery for SPNs between September 1998 and July 2020. Results: Surgical intervention, typically required for symptomatic cases or pathological confirmation, yielded favorable outcomes with a 5-year survival rate of up to 97%. Despite challenges in standardizing preoperative evaluation and follow-up protocols, aggressive complete resection showed promising long-term survival and good oncological outcomes. Notably, no significant differences were found between conventional and minimally invasive (MI) surgery in perioperative outcomes. Histopathological correlations were lacking in prognosis and locations. Among the patients, one developed diffuse liver metastases 41 months postoperatively but responded well to chemotherapy and transcatheter arterial chemoembolization, with disease stability observed at 159 postoperative months. Another patient developed nonalcoholic steatohepatitis after surgery and underwent liver transplantation, succumbing to poor medication adherence 115 months after surgery. Conclusions: These findings underscore the importance of surgical intervention in managing SPNs and suggest the MI approach as a viable option with comparable outcomes to conventional surgery.

Robot-assisted distal pancreatectomy with spleen preservation in the treatment of a child with a solid pseudopapillary tumor

The use of robots in pancreatic surgery offers several advantages as an adjunct to laparoscopic surgery, including increased maneuverability of robotic instruments and three-dimensional (3D) visualization. To our knowledge, only two cases of robot-assisted distal pancreatectomy with spleen preservation in children have been reported worldwide. In this study, the patient was an 11-year-old boy who was admitted to a children’s hospital with complaints of recurrent upper abdominal pain. He was diagnosed with pancreatic tumor based on ultrasound findings. It was confirmed by magnetic resonance imaging, which revealed a well-defined heterogeneous formation at the border of the body and tail of the pancreas, measuring 2.28 × 2.73 × 2.62 cm with compression of the Wirsung duct. The surgical intervention was performed using a surgical robot VERSIUS (CMR, (UK). Splenic vessels were mobilized carefully, a tourniquet was placed around the body of the pancreas, and it was intersected using a stapler at the border of healthy tissue. The surgical intervention took 340 min, including robot installation time (docking time) of 15 min and a main console time of 325 min. No serious intraoperative complications, such as bleeding or damage to the vascular structures adjacent to the pancreas (branches of the celiac trunk and portal vein), were noted. Histological examination of the tumor confirmed the diagnosis of a solid pseudopapillary tumor. After surgery, the patient’s condition was stable. Recovery proceeded without complications. Magnetic resonance imaging of the abdominal cavity, which was performed 6 months after surgery, revealed no signs of disease recurrence. Robot-assisted surgery is an acceptable alternative to laparoscopic and open surgery for patients with solid pseudopapillary pancreatic tumors because robots offer additional connections, enable closure in improved 3D imaging, increase dexterity when handling instruments, and eliminate of hand tremors.

A Rare Co-existent Case of Splenic Microfilariasis and Pancreatic Solid Pseudopapillary Epithelial Neoplasm - A Double Jeopardy!

Filariasis is a major public health concern in tropical and subtropical countries like India with Wuchereria bancrofti accounting for 90% of lymphatic filariasis. Rarely observed are extra lymphatic manifestations caused by interaction of immune system with microfilaria and their diffusible products. Among various organs involved, splenic involvement is a rare extra lymphatic manifestation of filariasis and can masquerade clinicoradiologically as metastasis when associated with a known malignancy or as a primary malignancy like lymphoma. Hereby, we present an unusual case of coincidence of splenic filariasis with pancreatic solid pseudopapillary epithelial neoplasm in a 20-year-old woman associated with peripheral blood eosinophilia.

Frantz Tumor, a Rare Indolent Pancreatic Neoplasm Entity: A Case Report and Brief Review

Solid pseudopapillary neoplasm (SPN) of the pancreas, also known as Frantz tumor, is an uncommon tumor with indolent clinical features that primarily affects young women. We are presenting a 27-year-old Caucasian female with an accidental finding with pancreatic SPN who underwent complete resection of the tumor using a distal pancreatectomy and splenectomy procedure. Immunohistochemistry revealed low-grade pancreatic SPN. Despite the rare appearance, it should be considered in the differential diagnosis of a young female with a large pancreatic mass.

High-grade Solid Pseudopapillary Neoplasms of the Pancreas: Distinct Clinicopathological Malignant Features With Intriguing Gene Alterations through a Comparison With the Conventional Type.

Pancreatic solid pseudopapillary neoplasm (SPN) is a low-grade malignant neoplasm with a good prognosis. Clinically aggressive SPNs have rarely been reported but have not been analyzed in detail. In this study, we referred to this highly malignant type of SPN as high-grade SPN (HG-SPN) and compared its clinicopathological and genetic characteristics with conventional SPN (C-SPN) using immunohistochemistry and gene panel analyses. Five HG-SPNs and 15 C-SPNs were evaluated in this study. HG-SPNs share many pathologic characteristics: macroscopically, solid/cystic appearances, microscopically, pseudopapillary/pseudorosette pattern (100%), tumor cell loose cohesiveness (100%), thin/delicate vasculature (100%), tumor cell cytoplasmic vacuolization (100%), immunohistochemical positivity for β-catenin (nuclear expression) (100%), CD10 (80%), CD56 (80%), and vimentin (100%). Conversely, HG-SPNs showed distinct malignant features compared with C-SPNs: mean tumor size (11.7 vs. 2.9 cm, P <0.001); true necrosis (100% vs. 0%, P <0.001); high-grade nuclear atypia (100% vs. 0%, P <0.001); lymphatic and/or venous invasion (100% vs. 20%, P =0.004); mean mitotic count (4.38 vs. 0.05/high-power field, P <0.001); and mean Ki-67 labeling index (33.9% vs. 3.4%, P <0.001). All HG-SPN patients died of primary disease 3 to 36 months after surgery, while all C-SPN patients were alive without disease. Genetic studies have shown that all analyzed HG-SPNs have CTNNB1 mutations. Two HG-SPN cases showed RB1 mutations with altered immunohistochemical findings for RB1 and p16. Two HG-SPN cases had TP53 mutation and/or p53 overexpression. In conclusion, HG-SPNs show distinct malignant features and some genetic alterations that differ from C-SPNs, indicating the importance of differentiating between these 2 subtypes.

Total laparoscopic curative resection of a rare pan-necrotic solid pseudopapillary pancreatic tumor in a child

Abstract: Tumor necrosis is purportedly related to an aggressive tumor phenotype in few solid tumors such as pancreatic ductal carcinoma, breast and bladder carcinomas. We hereby report a unique pan-necrotic (98% of tumor) pseudopapillary pancreatic tumor in a 13-year-old child who underwent laparoscopic subtotal pancreatectomy and curative resection. To our knowledge, this is the first account of complete tumor necrosis in a pancreatic neoplasm with curative laparoscopic resection. This case report has discussed the literary background of various mechanisms of cell death in solid tumors to explain the plausible cause of unique pan-tumor necrosis.

Differential diagnosis between pancreatic solid pseudopapillary tumors and pancreatic neuroendocrine tumors based on contrast enhanced ultrasound imaging features.

To evaluate the application of contrast enhanced ultrasound (CEUS) in preoperatively differential diagnosis between pancreatic solid pseudopapillary tumors (SPTs) and pancreatic neuroendocrine tumors (pNETs). This retrospective study was approved by Institutional Review Board. Patients with surgical resection and histopathological diagnosis as SPTs and pNETs were included. All patients underwent B mode ultrasound (BMUS) and CEUS examinations within one week before surgical operation. On BMUS, the size, location, echogenicity, calcification, and margin of lesions were observed and recorded. On CEUS imaging, enhancement patterns, and enhancement degrees were recorded and analyzed. An independent t-test or Mann-Whitney U test was used for comparison between continuous variables. Chi-square test was used to compare the CEUS patterns. From February 2017 to Dec 2022, patients diagnosed as SPTs (n = 39) and pNETs (n = 48) were retrospectively included. On BMUS, anechoic cystic changes (19/39, 48.72%) and hyperechoic calcification (14/39, 35.90%) are more commonly detected in SPTs (P = 0.000). On CEUS imaging, the majority of SPTs (27/39, 69.23%) showed hypo-enhancement in the arterial phase, while most of the pNETs (36/48, 75.00%) showed hyper- or iso-enhancement in the arterial phase (P = 0.000). In the venous phase, most of the SPTs (32/39, 82.05%) showed hypo-enhancement, while over half of pNETs (29/48, 60.42%) showed hyper- or iso-enhancement compared to pancreatic parenchyma (P = 0.001). CEUS is a valuable and non-invasive imaging method to make preoperatively differential diagnoses between SPTs and pNETs.

High frequency of alternative splicing variants of the oncogene Focal Adhesion Kinase in neuroendocrine tumors of the pancreas and breast.

The characteristic genetic abnormality of neuroendocrine neoplasms (NENs), a heterogeneous group of tumors found in various organs, remains to be identified. Here, based on the analysis of the splicing variants of an oncogene Focal Adhesion Kinase (FAK) in The Cancer Genome Atlas datasets that contain 9193 patients of 33 cancer subtypes, we found that Box 6/Box 7-containing FAK variants (FAK6/7) were observed in 7 (87.5%) of 8 pancreatic neuroendocrine carcinomas and 20 (11.76%) of 170 pancreatic ductal adenocarcinomas (PDACs). We tested FAK variants in 157 tumor samples collected from Chinese patients with pancreatic tumors, and found that FAK6/7 was positive in 34 (75.6%) of 45 pancreatic NENs, 19 (47.5%) of 40 pancreatic solid pseudopapillary neoplasms, and 2 (2.9%) of 69 PDACs. We further tested FAK splicing variants in breast neuroendocrine carcinoma (BrNECs), and found that FAK6/7 was positive in 14 (93.3%) of 15 BrNECs but 0 in 23 non-NEC breast cancers. We explored the underlying mechanisms and found that a splicing factor serine/arginine repetitive matrix protein 4 (SRRM4) was overexpressed in FAK6/7-positive pancreatic tumors and breast tumors, which promoted the formation of FAK6/7 in cells. These results suggested that FAK6/7 could be a biomarker of NENs and represent a potential therapeutic target for these orphan diseases.

Comprehensive study of clinical features, prognostic factors, and survival in patients with pancreatic solid pseudopapillary neoplasms based on the 2019 WHO classification.

Pancreatic solid pseudopapillary neoplasms (pSPNs) are a rare tumor type with a limited understanding of their clinical characteristics and survival outcomes. We aimed to investigate potential prognostic factors among the existing clinical features in patients diagnosed with pSPN. For this study, we utilized data from the Surveillance Epidemiology and End Results (SEER) database, specifically selecting patients with a histology type of pSPN from the years 2000 to 2019. Subsequently, we conducted both univariate and multivariate Cox regression analyses in a systematic manner to identify potential prognostic factors associated with overall survival (OS) and cancer-specific survival (CSS) in the selected group of patients. To assess the disparity in OS and CSS among different clinical features and treatments, Kaplan-Meier curves were generated. Furthermore, utilizing the results obtained from the multivariate analysis, we developed a nomogram predictive model to effectively forecast the prognosis of patients diagnosed with pSPN. Calibration plots were presented to demonstrate the predictive accuracy and reliability of the nomogram predictive model. The study comprised a total of 433 participants, with 85.7% of the patients diagnosed with pSPN being female and the remaining 14.3% being male. The Kaplan-Meier curves indicated that patients with pSPN who underwent primary tumor resection (PTR) and those who were younger than 70years old had significantly improved OS and CSS compared to those who did not undergo PTR or were aged 70years or older, respectively (P < 0.001). Male patients diagnosed with pSPN exhibited poor OS compared to female pSPN patients (P = 0.015). The multivariate Cox regression analysis indicated that age (OS: HR = 1.055, 95% CI = 1.027-1.084, P < 0.001. CSS: HR = 1.054, 95% CI = 1.019-1.091, P = 0.002) and PTR (OS: HR = 6.074, 95% CI = 1.922-19.194, P = 0.002. CSS: HR = 4.912, 95% CI = 1.188-20.312, P = 0.028) were independent prognostic factors for both OS and CSS. Moreover, tumor size (≥ 5 vs < 5cm CSS: HR = 4.788, 95% CI = 1.012-22.661, P = 0.048) was an independent prognostic factor for CSS. The independent prognostic factors identified through the multivariate Cox regression analysis were utilized to construct a nomogram model for predicting both OS and CSS in patients with pSPN. The accuracy of the nomogram model was visually testified by the calibration plot with acceptable predictive performance. Although the majority of patients diagnosed with pSPN are females, it was observed that male patients tend to have poorer OS compared to their female counterparts. The independent prognostic factors identified in the study were age and PTR, which were associated with both OS and CSS. Tumor size was an independent prognostic factor for CSS. Patients who underwent PTR exhibited improved OS and CSS outcomes. The developed nomogram and corresponding reference table provided promising prognostic predictions for pSPN outcoms, serving as a valuable resource for clinicians and patients alike.

Case of Treatment of Solid Pseudopapillary Pancreatic Tumor

Introduction. A solid pseudopapillary pancreatic tumor accounts for less than 3 % of all pancreatic tumors. It is more common in young women aged 20 to 30. The prognosis for this pathology is favorable. Complete removal of the tumor leads to full recovery in more than 85 % of patients. Materials and methods. The paper presents a clinical case which demonstrates the surgical outcome of a patient with a malignant pancreatic tumor. Patient M., female, born in 1998, underwent inpatient treatment at the I.K. Akhunbaev Clinic of National Hospital, Kyrgyzstan, from January 12, 2015 to January 26, 2015 with a clinical diagnosis — pancreatic head mass, which was found intraoperatively. Due to this, the decision was made to expand the scope of the surgery. Histopathological examination revealed a solid pseudopapillary tumor. Results and discussion. This tumor is extremely rare and is usually detected accidentally during preventive examinations, or when the tumor becomes large. Against the background of treatment, the dynamics of observation for 7 years revealed no signs of progression and relapse of this pathology. Conclusion. Solid pseudopapillary tumor is a rare, highly differentiated malignancy, which develops most oft en in young women. It is characterized by a relatively favorable clinical course, which is demonstrated by this case report.

The first case reported in Southeast Asia of Huge Frantz’s Tumor

Introduction: Solid pseudopapillary pancreatic tumor (Frantz’s Tumor) is a low-grade malignancy, that lacks clinical symptomatology. It was treatable with a favorable prognosis. Case presentation: A young female patient presented with increasing abdominal pain three years ago. Abdominal examination revealed a lump palpable. Contrast-enhanced Computed Tomography abdomen reported a solid mass measuring 13.9 cm x 13.2 cm x 8.9 cm from the pancreas with an enhancing cystic component. Fine-needle aspiration biopsy yielded a cellular sample comprising pseudopapillary neoplasm of the pancreas. A distal pancreatectomy was performed; she was discharged in satisfactory condition.

Organ-preserving surgery and classic surgery for pancreatic solid pseudopapillary neoplasms: a multicenter analysis from Central and Western China

Background: Pancreatic solid pseudopapillary neoplasms (SPNs) are low-grade malignant tumors of the pancreas. Organ-preserving surgery is being increasingly performed for these tumors, although there is little evidence to support its use. This retrospective multicenter study aimed to determine the clinicopathologic characteristics of pancreatic SPNs in Central and Western China and to determine the efficacy of organ-preserving surgery. Methods: The clinicopathologic, treatment and follow-up data of 227 pancreatic SPN patients treated between July 2003 and December 2016 at 5 tertiary care centers were retrospectively reviewed. Results: Among the 227 patients (38 males, 189 females; mean age 33.30 ± 12.70 years), only 72 (31.7%) had symptoms. The mean tumor size was 55.08 ± 29.56 mm. Adjacent organ/lymphovascular invasion was present in 16 (7.1%) patients and distant metastasis in 3 (1.3%) patients; no patient had spread to distant lymph nodes. Surgery included organ-preserving surgery in 108 (47.6%) patients, classic surgery in 115 (50.7%) patients, and palliative surgery in 4 (1.8%) patients. Tumor recurrence was seen in 8 (3.6%) patients. Cox regression analysis showed positive surgical margin (P &lt; .01) and metastasis (P = .03) to be independent predictors of recurrence. Tumor characteristics were comparable between patients receiving organ-preserving surgery and classic surgery. The risk of recurrence was also similar in both groups (P = .72). Conclusions: Organ-preserving surgery appears to be an effective procedure for the treatment of pancreatic SPNs. Resection surgery should aim for negative surgical margins.

Can solid pseudopapillary neoplasm of the pancreas without degeneration be diagnosed with imaging? a comparison study of the solid component of solid pseudopapillary neoplasm, neuroendocrine neoplasm, and ductal adenocarcinoma.

To investigate the MR findings of the solid components within pancreatic solid pseudopapillary neoplasms (SPNs) to characterize solid SPN without degeneration. After case matching, 23 patients with SPNs, 23 with pancreatic neuroendocrine neoplasms (PNENs), and 46 pancreatic ductal adenocarcinomas (PDACs) were included in this retrospective comparative study. The MR findings of the solid components within the pancreatic tumors were assessed qualitatively and semi-quantitatively. In the qualitative assessment, significant differences were noted in T2-weighted imaging and MR cholangiopancreatography (MRCP). SPNs with a score of 4-5 (iso- to hyper-intense compared with the renal cortex) were observed in 18/19 (94.7%) by reader 1 and 15/19 (78.9%) by reader 2 (score 5, 52.6% and 47.4%) on fast spin-echo (FSE) T2-weighted imaging. On MRCP, the two readers identified 12 (63.2%) and 8 (42.1%) SPNs, respectively. The semi-quantitative signal-intensity ratio (SIR, signal intensity of tumor/signal intensity of the pancreatic parenchyma) of SPNs on FSE T2-weighted imaging was significantly higher (mean, 1.99-2.01) than that of PNENs (1.30-1.31) or PDACs (1.26-1.28). The sensitivity/specificity of 'hyper' on T2-weighted imaging (qualitative score of 4-5, or SIR of ≥ 1.5) were 78.9-100.0%/63.8-79.7%. The sensitivity/specificity of 'remarkably hyper' (score of 5, SIR of ≥ 2.0, or visible on MRCP) or salt-and-pepper pattern were 36.8-68.4%/85.5-98.6%. T2-weighted imaging may be the key sequence for solid SPN. Solid tumors with hyper-intensity on T2-weighted imaging (especially, more hyper-intense than the renal cortex, more than twice the signal of the pancreatic parenchyma, depicted on MRCP, or salt-and-pepper appearance) may be suspected to be SPNs.

Solid pseudopapillary neoplasm, a rare pancreatic tumor-case report

The uncommon cystic exocrine pancreatic tumor known as solid pseudopapillary epithelial neoplasm (SPEN) most frequently affects young females. We provide a case study of a 17-year-old female who experienced intermittent vomiting and epigastric pain. Her laboratory results were within normal ranges, and a clinical abdominal exam revealed pain in the region of epigastrium. A 40×3×31 mm iso-hyperdense minimally enhancing solid mass with well-defined borders was detected on her CECT scan. Nothing calcified. neither localized lymphadenopathy nor fat stranding. It was just next to the splenic vein, which was completely opaque. Her histopathology revealed a solid pseudopapillary pancreatic tumor after she underwent a spleen preserving distal pancreatectomy. Beta-catenin and vimentin IHC assays were significantly positive, confirming the diagnosis and she was on a regular follow-up. As of now, the patient is asymptomatic and has not experienced a recurrence. In contrast to other forms of pancreas tumors, SPEN is treatable with early identification and full surgical resection.

Gastrointestinal: Abdominal eggshell sign in a young woman with a pancreatic solid pseudopapillary neoplasm.

Gastrointestinal: Abdominal eggshell sign in a young woman with a pancreatic solid pseudopapillary neoplasm.

Multicenter Study on the Performance of Imaging Tests Compared to Endosonography-Guided Fine-Needle Aspiration in the Diagnosis of Solid Pseudopapillary Neoplasms of the Pancreas

Introduction: Imaging diagnosis of pancreatic solid-pseudopapillary neoplasms (SPNs) is difficult. Preoperative diagnosis by endosonography-guided fine-needle aspiration (EUS-FNA) is possible and has been reported in the literature in pancreatic tumors. However, its usefulness is still controversial. The aim of this study was to determine the accuracy of the EUS-FNA in the diagnosis of patients with SPN and describe the findings in computerized tomography (CT), magnetic resonance cholangiopancreatography imaging (MRI/MRCP), and EUS therefore comparing the imaging methods alone to the findings of microhistology (McH) obtained by EUS-FNA. Materials and Methods: We retrospectively reviewed the medical records of patients undergoing EUS-FNA with suspected SPN in imaging studies in 5 Brazilian high-volume hospitals (two university hospitals and three private hospitals). The demographic data; findings in CT, MRI/MRCP, and EUS; and McH results obtained by EUS-FNA were noted prospectively. The final diagnosis was obtained after the anatomopathological examination of the surgical specimen in all patients (gold standard), and we compared the results of CT, MRI/MRCP, EUS, and the McH with the gold standard. Results: Fifty-four patients were included in the study, of which 49 (90.7%) were women with an average age of 33.4 (range 11–78) years. The most common symptom presented was abdominal pain, present in 35.2% patients. SPN was detected incidentally in 32 (59%) patients. The average size of the tumors was 3.8 cm (SD: 2.26). The most common finding at EUS was a solid, solid/cystic, and cystic lesion in 52.9%, 41.1%, and 7.8% patients, respectively. The final diagnosis was 51 patients with SPN and 3 with nonfunctioning pancreatic neuroendocrine tumors (NF-NET). The correct diagnosis was made by CT, MRI/MRCP, EUS isolated, and EUS-FNA in 21.9%, 28.88%, 64.71%, and 88.24%, respectively. EUS-FNA associated with CT and MRI increased diagnostic performance from 22.72% to 94.11% and from 29.16% to 94.11%, respectively. Conclusions: SPN are rare, incidentally identified in most cases, and affect young women. Differential diagnosis between SPN, NF-NET, and other types of tumors with imaging tests can be difficult. EUS-FNA increases preoperative diagnosis in case of diagnostic doubt and should be used whenever necessary to rule out NF-NET or other type of solid/cystic nodular lesion of the pancreas.

Preoperative prediction of invasive behavior of pancreatic solid pseudopapillary neoplasm by MRI-based multiparametric radiomics models.

A log-combined model was developed to predict the invasive behavior of pancreatic solid pseudopapillary neoplasm (pSPN) based on clinical and radiomic features extracted from multiparametric magnetic resonance imaging (MRI). A total of 111 patients with pathologically confirmed pSPN who underwent preoperative plain and contrast-enhanced MRI were included, and divided into an invasive group (n = 34) and non-invasive group (n = 77). Clinical features and laboratory data related to pSPN invasive behavior were analyzed. Regions of interest were delineated based on T1-weighted imaging (T1WI), T2-weighted imaging (T2WI), diffusion-weighted imaging (DWI), and contrast-enhanced T1WI (CE-T1WI) to extract radiomic features. Correlation analysis was performed for these features, followed by L1_based feature selection (C = 0.15). A logistic regression algorithm was used to construct models based on each of the four sequences and a log-combined model was used to integrate the sequences. A receiver operating characteristic (ROC) curve was plotted to evaluate the model performance, and the Brier score was used to assess the overall accuracy of the model predictions. The area under the ROC curve was 0.68, 0.73, 0.71, and 0.49 for Log-T1WI, Log-T2WI, Log-DWI, and Log-CE models, respectively, and 0.81 for the log-combined model. The accuracy, precision, sensitivity, and specificity of the log-combined model were 0.77, 0.88, 0.75, and 0.78, respectively. The best performance was obtained with the log-combined model with a Brier score of 0.18. Tumor location was identified as a significant clinical feature in comparison between the two groups (p < 0.05), and invasive pSPN was more frequent in the tail of the pancreas. The log-combined model based on multiparametric MRI and clinical features can be used as a non-invasive diagnostic tool for preoperative prediction of pSPN invasive behavior and to facilitate the development of individualized treatment strategies and monitoring management plans.

Solid Pseudopapillary Neoplasm - Case Series and Review of Literature.

Solid-pseudopapillary neoplasm (SPN) is a variety of solid and cystic tumors of the pancreas. It was first described by Frantz in 1959. It is an unusual form of pancreatic carcinoma, with unknown etiopathogenesis, which accounts for about 0.17 to 2.7% of all pancreatic tumors. Here, we are describing 5 cases of pancreatic solid pseudopapillary neoplasm, out of 180 pancreatic tumors, operated in our institution in the 5-year period (2015-2020). Also, we have reviewed all available case series (from 2006 to 2020) in the literature, of pancreatic pseudopapillary neoplasm, for demographic information, etiopathogenesis, diagnosis, and extent of operation to establish the optimal management of this condition. Retrospective analysis of pancreatic tumors was carried out from February 2015 to January 2020. A total of 180 patients underwent pancreatic resection in this period for pancreatic tumor, out of which, the solid pseudopapillary neoplasm was confirmed in 5 cases (2.76%). Among these 5 cases, 4 cases (80%) were female and one (20%) male, with age group range from 14 to 45years (mean age - 28years). Abdominal pain was the most frequent presenting symptom (60%). Mean tumor diameter was 6.9cm (range, 2-18cm). Two patients were diagnosed preoperatively by CECT and MRI findings, and three patients were diagnosed preoperatively by percutaneous/USG-guided and CT-guided FNA cytology. Two patients underwent pancreatoduodenectomy; one patient underwent enucleation; and two patients underwent spleen preserving distal pancreatectomy. Four patients are alive and on regular follow-up, while one patient died on the 5th post-operative day due to post-operative sepsis.

Nonalcoholic fatty liver disease developed after pancreatoduodenectomy for solid pseudopapillary neoplasm in a 10-year-old girl: a case report

BackgroundSolid pseudopapillary neoplasms of the pancreas are rare. Moreover, pancreatoduodenectomy (PD) and postoperative care are not common in pediatric surgery. Herein, we report a case of PD and nonalcoholic fatty liver disease (NAFLD) after PD and present a literature review.Case presentationA 10-year-old girl with a suspected liver tumor was referred to our hospital. Echography, enhanced computed tomography and magnetic resonance imaging showed that the tumor coexisted with the solid and cystic parts of the pancreatic head. Since the patient was a young woman and the imaging findings were consistent with that of pancreatic solid pseudopapillary neoplasms (SPNs), we diagnosed her with pancreatic SPN. Thereafter, PD was performed, and she was discharged 10 days after the operation. Although her postoperative course was mostly uneventful, she experienced few episodes of abdominal pain and diarrhea before hospital discharge. These symptoms subsequently became more frequent and severe. The patient was urgently readmitted to the hospital for watery steatorrhea and lower abdominal colic pain. Her serum aspartate aminotransferase and alanine aminotransferase levels were elevated, and a fatty liver was detected on echography. The patient was diagnosed with steatorrhea, peristaltic pain, and NAFLD after PD. Pancrelipase (containing pancreatic digestive enzymes), antidiarrheal agents, and probiotics were started. Dosage increase of these drugs reduced the defecation frequency and abdominal pain and switched diarrhea to loose stools. However, more lipids in meals or more meals caused diarrhea and abdominal pain. Therefore, the doses of these drugs were further increased, and another antidiarrheal agent, loperamide hydrochloride, was added. Exocrine pancreatic enzymes supplementation and careful follow-up should prevent NAFLD progression after PD. At present, the patient has occasional abdominal pain, but has tangible soft stools once or twice a day. Although echography still shows a mottled fatty liver, her hepatic enzymes are only mildly elevated.ConclusionsPediatric PD is rare, and residual pancreatic function is usually sufficient, unlike in adult cases. However, we experienced a case of NAFLD after PD for a pediatric pancreatic SPN, in which pancreatic enzyme supplementation effectively improved this condition. Further attention must be paid to worsening of NAFLD that can develop nonalcoholic steatohepatitis.

NEOPLASIA SÓLIDA PSEUDOPAPILAR PANCREÁTICA MIMETIZANDO TUMOR NEUROENDÓCRINO DE BAIXO GRAU

Introduction: Solid pseudopapillary neoplasms (SPN) and pancreatic neuroendocrine tumors (NET) are rare diseases that are generally incidental findings in imaging tests. SPN is a low-grade tumor with good prognosis that more commonly affects young female patients. Pancreatic NET has a significant variability in outcomes, with low malignant potential in non-progressing tumors that are welldifferentiated. Both tumors may appear very similar in imaging tests and immunohistochemical (IHC) evaluation, which makes the differential diagnosis challenging, especially in small lesions. Case report: The authors present the case of a 34-year-old male with a medical history of a primary mediastinal germ cell tumor. The patient had no symptoms. Follow-up abdominal CT scan evidenced a single, well-delimited nodular lesion in the pancreatic neck, measuring 17mm in diameter. The patient was submitted to an endoscopic ultrasound-guided fine needle aspiration biopsy (EUS-FNA) and after IHC analysis, there was a diagnostic suspicion of low-grade pancreatic NET. Central pancreatectomy (CP) was performed and complete lesion analysis evidenced a pancreatic SPN. Conclusion: The SPN can mimic low-grade nonfunctioning pancreatic NETs in imaging tests and EUSFNA IHC evaluation.